During the past three to four decades, the incidence of acquired sensorineural hearing loss
(SNHL) in children living in more developed countries has fallen, as a result of improved …

Sáez, Juan C., Viviana M. Berthoud, María C. Brañes, Agustín D. Martínez, and Eric C.
Beyer. Plasma Membrane Channels Formed by Connexins: Their Regulation and Functions …

Objectives/Hypothesis To perform a systematic review of GJB2‐associated hearing loss to
describe genotype distributions and auditory phenotype. Data Sources 230 primary studies …

Hearing loss is a common and complex condition that can occur at any age, can be inherited
or acquired, and is associated with a remarkably wide array of etiologies. The diverse …

Hearing impairment (HI) affects 1 in 650 newborns, which makes it the most common
congenital sensory impairment. Despite extraordinary genetic heterogeneity, mutations in …

Background Mutations in the GJB2 gene cause one form of nonsyndromic recessive
deafness. Among Mediterranean Europeans, more than 80 percent of cases of …

Background Inherited hearing impairment affects about 1 in 2000 newborns. Up to 50
percent of all patients with autosomal recessive nonsyndromic prelingual deafness in …

Despite the enormous heterogeneity of genetic hearing loss, variants in one locus, Gap
Junction Beta 2 or GJB2 (connexin 26), account for up to 50% of cases of nonsyndromic …

Background DFNB1, the locus of an autosomal recessive form of deafness due to mutations
in the connexin-26 gene (CX26 or GJB2) is one of the most frequent hereditary defects in …

Congenital deafness accounts for about 1 in 1000 infants and approximately 80% of cases
are inherited as an autosomal recessive trait. Recently, it has been demonstrated that …